Felty syndrome in juvenile arthritis

Toomey, K.; Hepburn, Bonnie

doi:10.1016/s0022-3476(85)80299-7

Cited by 9 publications

(4 citation statements)

References 8 publications

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“…Splenomegaly is generally most prominent within the first years after onset of systemic onset disease. The degree of splenomegaly may be extreme, but it is uncommonly associated with Felty's syndrome (splenic neutropenia)85). Hepatomegaly is less common than splenomegaly.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis

Hahn

Kim

2010

Korean J Pediatr

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Juvenile rheumatoid arthritis (JRA) is the most common rheumatic childhood disease; its onset is before 16 years of age and it persists for at least 6 weeks. JRA encompasses a heterogeneous group of diseases that is classified according to 3 major presentations: oligoarthritis, polyarthritis, and systemic onset diseases. These presentations may originate from the same or different causes that involve interaction with specific immunogenetic predispositions, and result in heterogeneous clinical manifestations. An arthritic joint exhibits cardinal signs of joint inflammation, such as swelling, pain, heat, and loss of function; any joint can be arthritic, but large joints are more frequently affected. Extra-articular manifestations include high fever, skin rash, serositis, and uveitis. The first 2 types of JRA are regarded as T helper 1 (Th1) cell-mediated inflammatory disorders, mainly based on the abundance of activated Th1 cells in the inflamed synovium and the pathogenetic role of proinflammatory cytokines that are mainly produced by Th1 cell-stimulated monocytes. In contrast, the pathogenesis of systemic onset disease differs from that of other types of JRA in several respects, including the lack of association with human leukocyte antigen type and the absence of autoantibodies or autoreactive T cells. Although the precise mechanism that leads to JRA remains unclear, proinflammatory cytokines are thought to be responsible for at least part of the clinical symptoms in all JRA types. The effectiveness of biologic therapy in blocking the action of these cytokines in JRA patients provides strong evidence that they play a fundamental role in JRA inflammation.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis

Hahn

Kim

2010

Korean J Pediatr

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“…The incidence of Felty syndrome in patients with JRA is very low, thus we could not determine the frequency of positivity of RF in JRA. However, among previous cases of Felty syndrome developed in patients with JRA 2–4 , two showed RF negative, and one was RF positive. Furthermore, according to Bloom BJ et al 4 ,.…”

Section: Discussionmentioning

confidence: 86%

“…Although the pathogenesis is not clear, this syndrome is seen in only 1% of the patients with RA and is characterized by a high rheumatoid factor (RF) titer. It is rarely seen in patients with juvenile rheumatoid arthritis (JRA), with only three cases having been reported throughout the world 2–4 . Of those three cases, the syndrome developed during the childhood or adolescent period, but there have been no cases reported of it onsetting in adult patients.…”

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confidence: 99%

A case of adult‐onset Felty syndrome in juvenile rheumatoid arthritis

Lee

Han

Yoon

et al. 2005

Pediatrics International

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“…An increased risk for the development of lymphoproliferative malignancies in patients with Felty's syndrome has been reported [31]. While lymphadenopathy and splenomegaly are characteristic of systemic-onset JRA, Felty's syndrome is only uncommonly reported in JRA [51,52]. …”

Section: Clinical Featuresmentioning

confidence: 99%

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Prahalad

Glass

2002

Arthritis Res

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Chapter summaryThe chronic arthropathies of childhood share clinical and pathological features with rheumatoid arthritis (RA) in adults. Both are autoimmune diseases characterized by a destructive arthropathy. Both are likely to be complex genetic traits, with autoantibodies and with a type-1-T-helper-cell cytokine profile in disease tissues. In common with other autoimmune diseases, both have associations and linkage with human keukocyte antigen (HLA) genetic markers. However, there are also important points of distinction outlined in this chapter, which include substantial differences in clinical phenotype. Juvenile rheumatoid arthritis (JRA) is characterized by several subtypes, whereas RA is more homogeneous. There are differences in outcome: adults with RA tend to have a poorer outcome; in JRA, the outcome is more variable and can be predicted by phenotypes at presentation. In addition, patients with RA have a stronger family history of RA although both RA and JRA share an increased frequency of family history of other autoimmune diseases. The genetic markers present to variable degrees in the subtypes of JRA differ quite considerably from those of RA. This is particularly so with respect to HLA.

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Felty syndrome in juvenile arthritis

Cited by 9 publications

References 8 publications

Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis

Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis

A case of adult‐onset Felty syndrome in juvenile rheumatoid arthritis

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